Tetralogy of Fallot with Cor Triatriatum Dexter in an Adult Patient: A Case Report

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect with only approximately 3% of uncorrected patients surviving past age 40. In this case report, we present a 48‐year‐old mentally retarded man suffering from congenital spastic quadriplegia who was diagnosed with a unique combination of symptomatic TOF and cor triatriatum dexter (CTD). Reduced preload because of CTD with spastic quadriplegia that prevented physical exertion is believed to have facilitated this patient's unusually long survival.     

A case of complete situs inversus.

Complete situs inversus is a rare syndrome, with overall frequency estimated at 1/10,000 births, resulting from abnormal rotation of the cardiac tube during embryogenesis, of unknown mechanism. Recent studies suggest that left-right asymmetry defects are likely to be due to genetic abnormalities in the lefty, nodal, i.v., HAND, ZIC3, Shh, ACVR2B and/or Pitxz genes. In dextrocardia with situs inversus the heart is structurally normal in 90-95% of cases, in contrast to dextroversion (dextrocardia with situs solitus), which has a high incidence of structural cardiac defects. Atrial septal defect is one of the most common congenital cardiac anomalies in adults. Diagnosis is based on clinical manifestations and simple complementary diagnostic exams like abdominal and thoracic radiography and electrocardiogram. Prognosis in isolated dextrocardia depends on the congenital cardiac defects present. By contrast, in dextrocardia with situs inversus life expectancy is similar to that of the general population. The authors present the case of a 64-year-old German man admitted to the emergency care unit with a diagnosis of embolic stroke due to atrial fibrillation with fast ventricular rate. As clinical history could not be assessed due to language limitations, routine admission tests were performed. They revealed complete situs inversus with corrected ostium secundum atrial septal defect. Finally, the anatomic, pathologic, embryologic and etiologic features of complete situs inversus and related abnormalities of the cardiac structures are presented. Special emphasis is given to genetic abnormalities, the study of which has seen great advances since the 1990s thanks to new techniques of DNA analysis.     

A study of precordial leads in subjects with congenital dextrocardia and situs inversus viscerum

The chest leads of seven persons with congenital dextrocardia and situs inversus viscerum were studied. None of the seven showed any clinical evidence of heart disease. One subject, however, because she had systolic hypertension, was believed to be potentially abnormal, and was considered separately from the other six. The standard leads of this one subject were normal, but she had inverted T waves in CF_1–4 and diphasic T waves in CF₅ and CF₆. The data indicate that the chest leads of a person with congenital dextrocardia and situs inversus are approximately identical with those of an individual whose heart is on the left side, provided the technique of making the leads is altered to conform to the dextro-position of the heart.     

A Novel Conduit-Lengthening Technique to Facilitate the Arterial Switch Operation in an Infant with a Problematic Combination of Coronary Anomolies

We report the technical aspects of the arterial switch operation as we performed it in a 5-month-old infant who had situs inversus, dextrocardia, transposition of the great arteries, and inverted origin of the circumflex and right coronary arteries. The successful performance of this procedure required the use of a conduit-lengthening technique due to the much longer distance to the proposed site of coronary artery transfer.Key words: Abnormalities, multiple/surgery; cardiovascular surgical procedures/methods; coronary vessel anomalies/surgery; dextrocardia; heart defects, congenital/surgery; situs inversus; transposition of great vessels/surgeryThe arterial switch operation is the currently preferred management option for patients who have a diagnosis of d-transposition of the great arteries (d-TGA) with or without a ventricular septal defect (VSD), provided that left and right ventricular size and function are normal. The techniques of this operation are fairly standard, particularly in patients who have atrial and visceral situs solitus. However, there are few data about the intraoperative problems and available surgical approaches in patients who have d-TGA, dextrocardia, and situs inversus.^1,2 We describe the technical considerations of the arterial switch operation in one such patient.     

A mysterious case of fatal embolism after the repair of tetralogy of Fallot: secondary to arrhythmia?

Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease. The most important late complications observed after repair of TOF are atrio-ventricular arrhythmias and sudden cardiac death. We present a rare case of fatal embolism and sudden cardiac death in a 36-year-old patient, 30 years after the operation for TOF.     

Ivemark syndrome in association with congenital septum transversum defect and pancreatic divisum.

A four-month-old female baby presented with cyanosis and respiratory distress. A provisional diagnosis of congenital posterolateral diaphragmatic hernia was made but on exploration there was a defect in the septum transversum along with features of Ivemark syndrome - asplenia with visceroatrial heterotaxia, malrotation and pancreatic divisum - an association not yet reported in literature. The child did well after operative correction of the hernia. Echocardiography showed situs inversus with dextrocardia with double outlet right ventricle, atrial septal defect, ventricular septal defect, patent ductus arteriosus and pulmonary stenosis.     

Atrioventricular discordance without ventriculo-arterial discordance. Apropos of 3 cases

Three cases of atrioventricular without ventriculo-arterial discordance are reported. The first case was a young woman with situs solitus, atrioventricular discordance and ventriculo-arterial concordance, large atrial and small ventricular septal defects. The functional tolerance was relatively good. The second case was an infant girl with dextrocardia by dextroversion and abdominal situs solitus. The child had atrioventricular discordance with ventriculo-arterial concordance and an ostium primum atrial septal defect. Cyanosis was mild and, in addition, complete heart block was diagnosed on the fourth day of life. The functional tolerance was good in this case too and growth was normal. The third case was a young girl with levocardia, atrial situs inversus, and abdominal situs inversus. A double outlet right ventricle was diagnosed at catheterisation with atrioventricular discordance, ventricular septal defect, and severe valvular and infundibular pulmonary stenosis. The first palliative procedure was performed at eight months: a left Blalock-Taussig anastomosis. As cyanosis has recurred a second procedure is being considered, the child now being 5 years old. Different forms of atrioventricular discordance without ventriculo-arterial discordance have been described: atrioventricular discordance with ventriculo-arterial concordance; atrioventricular discordance with double outlet right ventricle; atrioventricular discordance with double outlet left ventricle; atrioventricular discordance with a single vessel issuing from the right or left ventricle. With reference to their personal cases and those described in the literature, the authors describe the anatomy, physiopathology and the clinical consequences of the different forms encountered in their cases. The different surgical techniques for each anatomical form of this condition are discussed.     

Transcatheter closure of perimembranous ventricular septal defect in a patient with situs inversus and dextrocardia

Successful transcatheter closure of a perimembranous ventricular septal defect with an Amplatzer device has been reported in patients with levocardia. We report a case in which the device could be deployed successfully in a child with isolated perimembranous ventricular septal defect with situs inversus and dextrocardia.     

Late right pulmonary artery stenosis after arterial switch operation in mirror image dextrocardia and situs inversus totalis

The operation of D-transposition of the great arteries is performed extremely rarely in patients with mirror image dextrocardia and situs inversus totalis. Therefore, it is not surprising that such unusual anatomy case presents problems with primary operation. We present a case of late right pulmonary artery stenosis after neonatal surgical anatomic correction of mirror image dextrocardia and situs inversus totalis.     

Repair and Follow‐up of Tetralogy of Fallot with Pulmonary Stenosis

Tetralogy of Fallot is the most common cyanotic congenital heart defect. Advances in surgical technique and postoperative care have improved survival which is now very good. Patients now face long‐term morbidities such as reduced exercise tolerance and arrthymias. Cardiologists caring for these patients are confronted with decisions regarding best care practices. This article will review the evidence available on repair and postoperative follow‐up for patients with Tetralogy of Fallot with pulmonary stenosis.     

Myocardial revascularization in dextrocardia with situs inversus]

BACKGROUND: The incidence of coronary artery disease in patients with dextrocardia associated with situs inversus is similar to that of the general population: Nevertheless, there are few papers regarding surgical myocardial revascularization in these patients. METHODS: We report two patients with dextrocardia and situs inversus who underwent myocardial revascularization by means of coronary artery bypass grafting, with the right internal mammary artery to the left anterior descending coronary artery in one case and with the left internal mammary artery as a free graft to the left anterior descending coronary artery associated with the repair of an aneurysm of the right coronary sinus of Valsalva in the second patient. RESULTS: There were no surgical complications; the patients were discharged on the 4th and 7th postoperative days, respectively. They are alive and well after 24 and 60 months of follow-up respectively. CONCLUSIONS: This report suggests that durable myocardial revascularization in dextrocardia with situs inversus can be successfully achieved with right or left mammary artery bypass grafting to the left anterior descending coronary artery. The advantage of using an in situ mammary artery is discussed.     

Prenatal diagnosis of tetralogy of Fallot associated with pulmonary artery sling: Two case reports

Tetralogy of Fallot (TOF) is a common condition accounting for 10%–20% of all fetal cyanotic congenital heart disease cases. Pulmonary artery sling (PAS), or aberrant left pulmonary artery, is a rare congenital cardiovascular malformation. Approximately 58%–83% of PAS is associated with other cardiovascular malformations, TOF being rarest. The diagnosis of PAS is generally incidental or made at autopsy. Cases of prenatal diagnoses of TOF associated with PAS have not yet been reported. Here, we report two cases of TOF associated with PAS diagnosed prenatally in our hospital.     

Mitral valve replacement in a patient with situs inversus and dextrocardia

Dextrocardia is a rarely seen cardiac malposition, often associated with multiple and complex congenital cardiac anomalies. Valve surgery for acquired valvular lesions in dextrocardia with situs inversus is rare. A 65-year-old man was admitted to our department with palpitation and dyspnea. Chest X-ray showed dextrocardia, and echocardiography revealed severe mitral regurgitation due to rheumatic valve degeneration. Prosthetic mitral valve replacement using a transseptal approach via a median sternotomy was successfully performed in the patient.     

Surgical correction of tetralogy of Fallot in a seventy-five year old patient

Tetralogy of Fallot is a congenital heart disease which is mostly diagnosed and treated in infancy. In the literature there are some cases where the diagnosis was made in adults. This report describes the case of a seventy-five year old man who presents with a dilated and severely hypertrophic right ventricle, a ventricular septum defect, an overriding aorta and a severe infundibular stenosis in the right ventricular outflow tract. The diagnosis of an unrepaired Tetralogy of Fallot was made. A full surgical correction of the Tetralogy was performed and the patient received an implantable defibrillator, making him the oldest patient repaired for Tetralogy of Fallot.     

Complete situs inversus and bicuspid aortic valve stenosis

Patients with congenital heart disease are increasingly surviving to adulthood and present interesting challenges during cardiac catheterization. Situs inversus with dextrocardia is a type of cardiac malposition most likely to exist with a structurally normal heart. We present the case of a patient with complete situs inversus and bicuspid aortic valve stenosis, a hitherto unreported association. Prototypical findings in this cardiac malposition are illustrated for proper recognition with emphasis on technical tips for left and right heart catheterization.     

Familial dextrocardia, divergent strabismus and situs inversus of optic disc.

A family is described in which three of four siblings have major congenital cardiac defects in association with ocular abnormalities. The eldest sibling has isolated dextrocardia and the second has total situs inversus. The fourth child has both atrial and ventricular septal defects, with pulmonary hypertension and right ventricular hypertrophy. The mother and third sibling have normal hearts but exhibit a number of ocular defects. The principal ocular anomalies demonstrated in this family are divergent strabismus, bilateral situs inversus of the optic disc, and myopia. The hereditary aspect of these multiple findings is discussed.     

Mechanical reperfusion during acute myocardial infarction in a patient with dextrocardia

Mechanical reperfusion is the preferred treatment for acute ST-elevation myocardial infarction, we describe a case of successful primary angioplasty and stenting in a patient with dextrocardia and situs inversus. Dextrocardia with complete situs inversus is a rare condition, occurring in about 2 in 10,000 live births. However, ischemic heart disease and myocardial infarction have been reported in patients with dextrocardia, and hence the coexistence of myocardial infarction and dextrocardia is not unusual. The incidence of atherosclerosis in this group is not known, but is considered to be the same as that in the general population. There have been a few reports of percutaneous coronary intervention in these patients. We describe a case of primary angioplasty and stenting in a patient with dextrocardia and situs inversus and the electrocardiographic correlation of successful myocardial reperfusion.     

Acute anteroseptal myocardial infarction in a patient with dextrocardia

Dextrocardia with situs inversus is an uncommon congenital condition in which the major visceral organs are reversed. The clinical diagnosis and electrocardiographic localization of myocardial infarctions in these patients remain a great challenge. We report a case of a 64-year-old man known with dextrocardia and situs inversus totalis presenting with acute chest pain irradiating to the right arm. The admission and reversed “normalized” electrocardiogram are presented, allowing for correct diagnosis of an acute anteroseptal myocardial infarction. The present case emphasizes the importance of performing a reversed electrocardiogram in patients with dextrocardia.     

Multivalvular infective endocarditis in a tetralogy of fallot

Infective endocarditis (IE) is still a problem in patients with adult congenital heart disease. Tetralogy of Fallot (TOF) is one of the most important manifestations of congenital heart disease, which carries a high risk for the development of IE. We present an 18-year-old male with TOF complicated by an aggressive form of IE involving all cardiac valves.     

A case of unusual longevity of tetralogy of Fallot confirmed by cardiac catheterization.

We describe a 61-year-old woman with tetralogy of Fallot and dextrocardia with complete situs inversus. The functional status of this patient was New York Heart Association (NYHA) class II and the systemic blood pressure was 100/54 mmHg. The hematocrit was 54.4% and the arterial partial pressure of oxygen was 53 mmHg. On cardiac catheterization, both pulmonary valvular and infundibular stenoses were of equal severity and the aorta and main pulmonary artery were of equal size. We think that this patient has survived to this unusual age for tetralogy of Fallot because of unusually low systemic pressure and a proper balance between the ventricular septal defect and the pulmonary stenosis.